Introduction: In 1894, Giuseppe Muscatello described what we believe to be the only case of an occipitocervical encephalocele with a communication to the stomach. This case and its history and context compared to the Chiari 3 malformation as described 3 years earlier by Hans Chiari are presented.
Conclusions: Based on the uniqueness of this case, we propose the term Chiari 3.5 malformation be used to describe its anatomical derailment.
Keywords: Cervical encephalocele; Chiari 3 malformation; History.